The Paradox of Muscle Hypertrophy in Muscular Dystrophy - 11/01/12

Doi : 10.1016/j.pmr.2011.11.014

Joe N. Kornegay, DVM, PhD ^a,^b,^c,^d,^⁎ , Martin K. Childers, DO, PhD ^e, Daniel J. Bogan, BA ^a,^c,^d, Janet R. Bogan, BS ^a,^c,^d, Peter Nghiem, DVM ^f, Jiahui Wang, PhD ^g, Zheng Fan, MD ^b,^d, James F. Howard, MD ^b,^d, Scott J. Schatzberg, DVM, PhD ^h, Jennifer L. Dow, BS ^a,^c,^d, Robert W. Grange, PhD ⁱ, Martin A. Styner, PhD ^g,^j, Eric P. Hoffman, PhD ^f, Kathryn R. Wagner, MD, PhD ^k,^l
^a Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599, USA
^b Department of Neurology, School of Medicine, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599, USA
^c Gene Therapy Center, School of Medicine, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599, USA
^d Senator Paul D. Wellstone Muscular Dystrophy Cooperative Research Center, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599, USA
^e Section of PM&R, Department of Neurology, School of Medicine, Wake Forest University Health Sciences, Room 258, Dean Biomedical Research Building, 391 Technology Way, Winston-Salem, NC 27101, USA
^f Department of Integrative Systems Biology, George Washington University School of Medicine and Research Center for Genetic Medicine, Children’s National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, USA
^g Department of Psychiatry, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599, USA
^h Veterinary Emergency and Specialty Center of Santa Fe, 2001 Vivigen Way, Santa Fe, NM 87505, USA
ⁱ Department of Human Nutrition, Foods, and Exercise, Virginia Tech University, 321 Wallace Hall, Blacksburg, VA 24061, USA
^j Department of Computer Science, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599, USA
^k Center for Genetic Muscle Disorders, Kennedy Krieger Institute, 801 North Broadway, Baltimore, MD 21205, USA
^l Departments of Neurology and Neuroscience, The Johns Hopkins School of Medicine, 707 Broadway, Baltimore, MD 21205, USA

Corresponding author. School of Medicine, Campus Box 7525, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599.

Riassunto

Mutations in the dystrophin gene cause Duchenne and Becker muscular dystrophy in humans and syndromes in mice, dogs, and cats. Affected humans and dogs have progressive disease that leads primarily to muscle atrophy. Mdx mice progress through an initial phase of muscle hypertrophy followed by atrophy. Cats have persistent muscle hypertrophy. Hypertrophy in humans has been attributed to deposition of fat and connective tissue (pseudohypertrophy). Increased muscle mass (true hypertrophy) has been documented in animal models. Muscle hypertrophy can exaggerate postural instability and joint contractures. Deleterious consequences of muscle hypertrophy should be considered when developing treatments for muscular dystrophy.

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Keywords : Muscular dystrophy, DMD, GRMD, Muscle hypertrophy

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Variable muscle involvement and hypertrophy

Postural instability and contractures

Mechanisms contributing to muscle hypertrophy

Myostatin inhibition in muscle disease

Summary

Esportazione

Vol 23 - N° 1

P. 149-172 - Febbraio 2012 Ritorno al numero

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